Biotechnology Journal International

Aims: Hemoglobinopathies including HbE β thalassemia are associated with significant cardiac and pulmonary compromise in affected persons. The aim of this study was to evaluate the cardiac and pulmonary health of transfusion dependent 50 HbE β thalassemia patients of West Bengal.

Study Design: 50 transfusion dependent HbE-β thalassaemia patients were selected to evaluate the pulmonary hypertension as it is often associated with anemia.

Place and Duration of Study: Sample: Out Patient Department of Thalassaemia Control Unit, Imambara Sadar Hospital, Chinsurah, Hooghly, West Bengal, India between January, 2014 to December, 2015.

Methodology: We have evaluated the ECG, Echocardiography and Chest X ray to assess the cardiac function. Chest X ray and Spirometry (FVC, FEV1, FEV1%, PFC) were also monitored to evaluate the pulmonary obstruction.

Results: 68% patients showed severe to moderate pulmonary restriction, where as only 10% patients were found to have no significant pulmonary restrictions. The remaining 22% patients showed mild restriction. Other clinical abnormalities found on these HbE-β thalassaemia patents includes palpitation, chest pain, dyspnoea, edema feet and  investigation reveals pulmonary arterial hypertension (PAH), volume overload of left heart, pulmonary arterial enlargement, cardiomegaly and LVH strain pattern.

Conclusion: In this study, we have found that 68% of all patients showed reportable pulmonary restrictions. Cardiomegaly, volume overload of left heart and LVH strain pattern was found in 26%, 22% and 16% of all patients respectively. Overall, this study will help to get a clear cut and overall idea about the cardiac and pulmonary health of the HbE-β thalassaemia, which ultimately help in the prospective management of the related complications associated with the disease.